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Prolapsing Ureterocele as a Cause of Hematuria in an Infant
THE JOURNAL OF UROLOGY
Vol. 77, No. 4, April 1957 Printed 1·n U.S.A.
PROLAPSING URETEROCELE AS A CAUSE OF HEJVIATURIA IN AN INFANT MAJOR A. M. WILEY*
Ureterocele, or cystic enlargement of the vesical portion of the ureter, is an uncommon urological condition occurring typically in adult females. It is bilateral in 10 per cent of cases. The enlargement, the pathology of ·which has been the subject of a good deal of discussion, is the result of congenital or acquired narrowing of the ureteric orifice. There are two forrn_s of the condition: I) lVIucus form, in which the cyst is formed of vesical mucosa and 2) prolapsing form, where the cyst contains a layer of muscle in its wall and is assumed to be an intravesical prolapse of the entire ureter. Common symptoms resulting from ureterocele are recurrent ureteric pain, renal pain, hematuria and dysuria. Lechler (18:-34) was the first to describe the condition, which he discovered in a 3-month-old infant. English (1938) collected 15 additional cases while Lipmann Wolf (1898), Grosglik (1901) and Cohn (1904) were the first surgeons to diagnose and treat the condition by conventional means. Blum (1920), reviewing 100 cases, was the first to adequately describe the nature of ureterocele while Gutierrez (1939) gave a detailed account of it, describing nine different varieties. Macpherson (1942) added a further case and discussed the pathology. A female infant suffering from ureterocele recently came under the care of Army Medical Services in Kluang, Johore and a perusal of the available literature suggested that in this instance the mode of presentation of the condition was unique. The patient perished and postmortem study was possible only because amongst this Muslim community the child happened to be a Chinese orphan, and a girl, purchased at very low cost by its guardians who abandoned it when it was seen to be ill. CASE REPORT
Khoo Siew Bee was an orphan aged 2 months who had apparently been quite healthy until the date of admission on December 13, 1955. She then vomited and passed a number of loose motions. On examination she was found to be dehydrated. Her skin was covered in tiny red papules and she appeared to have been neglected. Temperature was normal. Some moist sounds could be heard at the right base and the abdomen was distended and tympanitic. Bowel sounds were heard. The vulva was discolored, the urethra was patulous and appeared to be partially prolapsed. Her urine was streaked with fresh blood and mucus. A provisional diagnosis of gastroenteritis was made associated ,vith general neglect and vulvitis. Her condition gave rise to little alarm and she was treated by regulated oral feeds. Streptomycin was given for her perineal "infection." She continued to pass blood stained urine and died suddenly a week later. * Present address: Medical Directorate, G. H. Q., Far East Land Forces,% G. P 0., Bingapore, Malaya. Accepted for publication Jul,· 19, 1956. 597
598
A. M. WILEY
FIG. 1. Diagram of postmortem appearances. Prolapsing ureterocele, dilated ureters and pelvic hydronephrosis of kidneys containing cortical cysts.
Postmortem findings: The abdomen was opened by a midline incision. The bowels appeared to be normal. Two enormously distended ureters were seen extending from a small and thickened bladder to apparently normal kidneys. When the bladder was opened it was found to be partially filled by a delicate mucous ureterocele which extended out through the external urethral orifice thereby giving rise to the erroneous diagnosis of prolapsing urethra. The intramural portion of the right ureter was enormously dilated. The left ureteric orifice was in its normal position and was of normal appearance. Both renal pelves were distended. Both kidneys contained a number of large cortical cysts. One of these was in communication with the renal pelvis on the right side. See figure 1. COMMENT
The rarity of this condition in infants and the mrnsual mode presentation lead to a failure in diagnosis. Vulvitis associated with neglect is not uncommon among infants seen here, added to which it is the common practice of local herbalists to apply irritant concoctions to any diseased part; this procedure was indeed suspected in this case.
PROLAPSING URETEROCELE
599
There can be no doubt that the narrowing of the right ureteric orifice was congenital. A cystic condition of the kidneys has been noted by Williams in patients who suffer from lower urinary obstruction starting in fetal life, but the association between cortical cysts of the kidneys and congenital stenosis of the ureter is obscure. It is presumed that the condition of the left ureter was the result of chronic obstruction at the internal urethral orifice brought about by the prolapsing right urethrocele. In conclusion, a note regarding treatment. This would have been of little avail in this case where renal dysfunction was already extensive. In suitable cases a urethrocele may be widely incised by the diathermy. Gutierrez thoroughly describes the treatment of urethrocele. My thanks are due to Lt. Col. J. Irvine, O.B.E., O.C. British Military Hospital, Kluang for permitting me to investigate and treat the patient. REFERENCES
Arno, I.: A Companion in Surgical Studies. Edinburgh: Livingstone, 1949. GUTIERREZ, R.: Surg., Gynec. & Obst., 68: 611, 1939. MACPHERSON, I.: Brit. J. Surg., 29: 294, 1942. WILLIAMS, D. I.: Ann. Roy. Coll. Surg., 14: 107, 1954.
Vol. 77, No. 4, April 1957 Printed 1·n U.S.A.
PROLAPSING URETEROCELE AS A CAUSE OF HEJVIATURIA IN AN INFANT MAJOR A. M. WILEY*
Ureterocele, or cystic enlargement of the vesical portion of the ureter, is an uncommon urological condition occurring typically in adult females. It is bilateral in 10 per cent of cases. The enlargement, the pathology of ·which has been the subject of a good deal of discussion, is the result of congenital or acquired narrowing of the ureteric orifice. There are two forrn_s of the condition: I) lVIucus form, in which the cyst is formed of vesical mucosa and 2) prolapsing form, where the cyst contains a layer of muscle in its wall and is assumed to be an intravesical prolapse of the entire ureter. Common symptoms resulting from ureterocele are recurrent ureteric pain, renal pain, hematuria and dysuria. Lechler (18:-34) was the first to describe the condition, which he discovered in a 3-month-old infant. English (1938) collected 15 additional cases while Lipmann Wolf (1898), Grosglik (1901) and Cohn (1904) were the first surgeons to diagnose and treat the condition by conventional means. Blum (1920), reviewing 100 cases, was the first to adequately describe the nature of ureterocele while Gutierrez (1939) gave a detailed account of it, describing nine different varieties. Macpherson (1942) added a further case and discussed the pathology. A female infant suffering from ureterocele recently came under the care of Army Medical Services in Kluang, Johore and a perusal of the available literature suggested that in this instance the mode of presentation of the condition was unique. The patient perished and postmortem study was possible only because amongst this Muslim community the child happened to be a Chinese orphan, and a girl, purchased at very low cost by its guardians who abandoned it when it was seen to be ill. CASE REPORT
Khoo Siew Bee was an orphan aged 2 months who had apparently been quite healthy until the date of admission on December 13, 1955. She then vomited and passed a number of loose motions. On examination she was found to be dehydrated. Her skin was covered in tiny red papules and she appeared to have been neglected. Temperature was normal. Some moist sounds could be heard at the right base and the abdomen was distended and tympanitic. Bowel sounds were heard. The vulva was discolored, the urethra was patulous and appeared to be partially prolapsed. Her urine was streaked with fresh blood and mucus. A provisional diagnosis of gastroenteritis was made associated ,vith general neglect and vulvitis. Her condition gave rise to little alarm and she was treated by regulated oral feeds. Streptomycin was given for her perineal "infection." She continued to pass blood stained urine and died suddenly a week later. * Present address: Medical Directorate, G. H. Q., Far East Land Forces,% G. P 0., Bingapore, Malaya. Accepted for publication Jul,· 19, 1956. 597
598
A. M. WILEY
FIG. 1. Diagram of postmortem appearances. Prolapsing ureterocele, dilated ureters and pelvic hydronephrosis of kidneys containing cortical cysts.
Postmortem findings: The abdomen was opened by a midline incision. The bowels appeared to be normal. Two enormously distended ureters were seen extending from a small and thickened bladder to apparently normal kidneys. When the bladder was opened it was found to be partially filled by a delicate mucous ureterocele which extended out through the external urethral orifice thereby giving rise to the erroneous diagnosis of prolapsing urethra. The intramural portion of the right ureter was enormously dilated. The left ureteric orifice was in its normal position and was of normal appearance. Both renal pelves were distended. Both kidneys contained a number of large cortical cysts. One of these was in communication with the renal pelvis on the right side. See figure 1. COMMENT
The rarity of this condition in infants and the mrnsual mode presentation lead to a failure in diagnosis. Vulvitis associated with neglect is not uncommon among infants seen here, added to which it is the common practice of local herbalists to apply irritant concoctions to any diseased part; this procedure was indeed suspected in this case.
PROLAPSING URETEROCELE
599
There can be no doubt that the narrowing of the right ureteric orifice was congenital. A cystic condition of the kidneys has been noted by Williams in patients who suffer from lower urinary obstruction starting in fetal life, but the association between cortical cysts of the kidneys and congenital stenosis of the ureter is obscure. It is presumed that the condition of the left ureter was the result of chronic obstruction at the internal urethral orifice brought about by the prolapsing right urethrocele. In conclusion, a note regarding treatment. This would have been of little avail in this case where renal dysfunction was already extensive. In suitable cases a urethrocele may be widely incised by the diathermy. Gutierrez thoroughly describes the treatment of urethrocele. My thanks are due to Lt. Col. J. Irvine, O.B.E., O.C. British Military Hospital, Kluang for permitting me to investigate and treat the patient. REFERENCES
Arno, I.: A Companion in Surgical Studies. Edinburgh: Livingstone, 1949. GUTIERREZ, R.: Surg., Gynec. & Obst., 68: 611, 1939. MACPHERSON, I.: Brit. J. Surg., 29: 294, 1942. WILLIAMS, D. I.: Ann. Roy. Coll. Surg., 14: 107, 1954.